Gastrointestinal stromal tumor gist is an uncommon disease, accounting for 0. A total of 10046 gist patients were collected in which 56 cases with an unknown tumor site were excluded. Dog1 antibodies are a sensitive and specific marker against gist positive for cd117 and cd34 and negative for cd117 and cd34. It develops when cells in the body divide and grow at an excessive rate. Gastrointestinal stromal tumors gists are the most common mesenchymal neoplasms of the gastrointestinal tract. Case report multiple, malignant, rapidly progressing mini. Gastrointestinal stromal tumor gist complicating a colonic. Management colorectal gastrointestinal stromal tumors. Pdf extra luminal colonic gastrointestinal stromal tumor. Various hypotheses have been proposed regarding the simultaneous development of gi. Synchronous occurrence of ileal stromal tumor gist and.
Colonic gastrointestinal stromal tumour presenting as intussusception simmi k ratan, garima goel, 1 parul sobti, 1 nita khurana, 1 mohit mathur, shandip k sinha, satish k aggarwal abstract. The symptoms of gastrointestinal stromal tumor depend on the site and. Nccn guidelines with nccn evidence blocks very advanced head and neck cancer about nccn evidence blocks nccn guidelines. Gastro intestinal stromal tumours are the uncommon tumours formed in the gastro intestinal tract. Tumor size, location and growth pattern are associated with symptomatic presentation 6. These tumours are formed in the early forms of special cells found in the wall of the gi tract, called the intestinal cells of cajal iccs. Protocol for the examination of specimens from patients. Image showing gastrointestinal stromal tumor after surgical removal localized, resectable adult gists, if anatomically and physiologically feasible, surgery is the primary treatment of choice. Here, we utilized singlecell rna and proteomic technologies to dissect the role of cafs on pdac cell heterogeneity in. Clinical and histopathological features of colonic stromal. Gastrointestinal stromal tumor is a disease in which abnormal cells form in the tissues of the gastrointestinal tract.
We report a 4yearold girl with a submucosal gist of the ascending colon, which was detected by computed tomography. E gist arising from the sac of the jejunal mesentery, and mimicking preoperatively as a colonic neoplasm is very rare. Gastrointestinal stromal tumors gists are undifferentiated tumors, usually diagnosed by immunohistochemistry. Gastrointestinal stromal tumor gist overview mayo clinic.
All gists have malignant potential, but the risk of this is minimal for very small. Gastrointestinal stromal tumors journal of gastrointestinal and. Gists of the colon and rectum constitute 5% of all cases and occur more often in the rectum 2 than. Gists of the colon and rectum constitute 5% of all cases. Our large database analysis aims to determine the characteristics of gists in the esophagus, colon and rectum and compare to gastric gists. If cancerous, the tumor may also be called a soft tissue sarcoma. Synchronous occurrence of colonic intraepithelial neoplasia. To the best of the authors knowledge, this is the first reported case of gist complicating a. Gastrointestinal stromal tumor of the colon endoscopic treatment by fullthickness resection a 51yearold man was referred for therapy of a submucosal tumor of the sigmoid colon. Gastrointestinal stromal tumors treatment adult pdq. Current clinical management of gastrointestinal stromal tumor ncbi.
Masthead postal and contact information volume 5 supplement 2 journal of the national comprehensive cancer network jnccn the jnccn issn 15401405, the official journal of the national comprehensive cancer network, is published 10 times annually by jones and bartlett publishers, 40 tall pine drive, sudbury, ma 01776. For gist tumors arising in the tubular organs of the digestive system esophagus, stomach, small intestine, appendix, colon and rectum, any extension beyond the muscular wall e. American cancer society, american society for colposcopy and cervical pathology, and american society for clinical pathology screening guidelines for the prevention and early detection of cervical cancer. Gist is the most common mesenchymal tumor of the gastrointestinal tract. The gastrointestinal and breast protocols have been updated to reflect the revised who histologic types. The college of american pathologists february 2020 release includes 47 revised cancer protocols and one new adult autopsy reporting protocol. Gastrointestinal stromal tumors gists are rare neoplasms of the gastrointestinal tract associated with high rates of malignant transformation. These tumors can also be other types of sarcoma that start in the blood vessels or connective tissue of the colon. Tumores del estroma gastrointestinal gist del colon. Most gastrointestinal stromal tumors gists occur in the stomach or small intestine. The gastrointestinal gi tract is part of the bodys digestive system. The incidence of gastrointestinal stromal tumors gists located in the colon is rare.
The incidence of allsite gists in the united states is estimated at 3300 to 4350 annually. Gastrointestinal stromal tumors gists of the colon. Pdf gastrointestinal stromal tumors gists of the colon. Most gists 95% express kit cd117, cd34 70%, and heavy caldesmon 80%, whereas 25% are positive for smooth muscle actin and less than 5% for desmin. These tumors often grow into the empty space inside the gastrointestinal gi tract, so they might not cause symptoms right away unless they are in a certain location or reach a certain size. Gastrointestinal stromal tumors home journal of gastrointestinal. Gastrointestinal stromal tumors gists are the most common mesenchymal neoplasms of the gastrointestinal tract, and typically present as discrete well. We report this case to describe the cytological features of gist and pitfalls in the cytology. We report here on an 80yearold male patient with advanced gist of the transverse colon.
Gastrointestinal stromal tumors gists are the most common malignant subepithelial lesions sels of the gastrointestinal tract. Colon, gastrointestinal stromal tumor, interstitial cells of cajal. Get an overview of gastrointestinal stromal tumors and the latest key statistics in the us. It is important to distinguish the diagnosis of lms from other gi mesenchymal tumors, particularly gist, as the. Gastrointestinal stromal tumours gist are the most common stromal or mesenchymal neoplasms effecting the gastrointestinal gi tract, representing less than 1% of all gi tumours, and over 90% of sarcomas 1 3. Gastrointestinal stromal tumors are the commonest mesenchymal tumors of the gastrointestinal tract, the stomach and small intestine are the favored sites of occurrence. While it has been found throughout the colon, it most commonly originates primarily from the sigmoid and transverse colon 4, 5. Leiomyosarcoma is a rare entity to arise primarily from the colon. However, apc in the incidence of colonic gist does not significantly change between 2000 and 2015. The following tests are generally ordered in the workup of patients who present with nonspecific abdominal symptoms.
Synchronous ileal stromal tumor gist and colonic adenocarcinoma. Jan 10, 2020 no laboratory test can specifically confirm or rule out the presence of a gastrointestinal stromal tumor gist. Gastrointestinal stromal tumor of the colon mimicking. Extra luminal colonic gastrointestinal stromal tumors are very rare and can present as mass abdomen. Gastrointestinal stromal tumor gist complicating a. A diagnostic mimicker of gastrointestinal stromal tumor article pdf available in case reports in gastroenterology 103. Stomach followed by small intestine is the most common sites of involvement, implicated in 95% of the cases. Large intestine and rectum constitute only 510% of git tumors. Gastrointestinal stromal tumor radiology reference.
Gastrointestinal stromal tumors gists are soft tissue sarcomas that can be located in any part of the digestive system. In this report, we describe a case of colonic perforation caused by an unusual form of gist. Dog1 is a novel gene on gastrointestinal stromal tumors gists that encodes the chloride channel protein anoctamin 1, also known as discovered on gist 1 dog1 protein. Gastrointestinal stromal tumor of the colon endoscopic.
To the best of the authors knowledge, this is the first reported case of gist complicating a colonic. We present a case of gist complicating a colonic interposition. The demographic characteristics are demonstrated in table 1. Therefore, the most common appearance is that of a mass arising from the wall of the gastrointestinal tract and projecting into the abdominal cavity. The way in which gist grows, or the route the tumor takes when it spreads through the body, is called its pathophysiology. Gastrointestinal stromal tumors gists are tumors, or clusters of overgrown cells, in the gastrointestinal gi tract. Endometriosis mimicking colonic stromal tumor the harvard community has made this article openly available. Gist has become a treatable disease, thanks to advances in research and. In the present case, as the colonic tumor was associated with peritoneal metastasis, lymph node metastasis might be a reflection of the high grade malignancy of this tumor. Although they may arise anywhere of gi tract, colonic presentation is infrequent. Diagnosis after ileocecal resection was established by histology. Types of colon cancer memorial sloan kettering cancer center. Molecularly targeted therapy with imatinib mesylate is the standard treatment for unresectable and metastatic gists. Colon excluding appendix, gastrointestinal stromal tumor, and neuroendocrine tumor primary site.
Their most common sites are the stomach and small intestine. Colon protocol has been updated requiring reporting of macroscopic evaluation of mesorectum. Gastrointestinal stromal tumors gists of the colon and rectum are the most common mesenchymal tumors of the gastrointestinal tract. Scientists are beginning to unravel some of the processes that go on inside cells that cause them to develop into gists.
Gastrointestinal stromal tumors smooth muscle tumors gists primary in the omentum and mesentery. To the best of our knowledge, the ct appearance of colon cancer which simulated the classic appearance of a gist has only been reported once in the worlds literature 2. Here we present colonic gist diagnosed as gist on cytology and confirmed on histopathology. The entity has a poorer prognosis than other gists. Stomach is the most common site 6065% of these tumors. Gist is neoplasm of mesenchymal origin originating from precursors of the interstitial cells of cajal. Extra luminal colonic gastrointestinal stromal tumor. Gastrointestinal stromal tumors can be a type of soft tissue sarcoma that can be found anywhere in the gastrointestinal tract but is rare in the colon. Gists preferentially develop in the stomach and the small bowel, although the tumors can arise anywhere in the digestive tract from the esophagus to the rectum. Tests for gastrointestinal stromal tumors stages and outlook prognosis after a cancer diagnosis, staging provides important information about the extent of cancer in the body and anticipated response to treatment. Sparse information is available about gists in uncommon locations. Gastro intestinal stromal tumours list of high impact. An exophytic adenocarcinoma of the colon is very rare with only a few reported cases 2, 3. In the literature a few cases of synchronous development of a gist and another neoplasia with.
Mucosal ulceration is seen on the luminal surface of the tumor in up to. It helps to digest food and takes nutrients vitamins, minerals, carbohydrates, fats, proteins, and water from food so they can be used by the body. Median age 56 years range 10 88 years associated with neurofibromatosis type 1 often with multiple tumors often long period to recurrence or metastases. The lesion had been diagnosed during screening colonoscopy, and biopsies had shown gastrointestinal stromal tumor gist.
Expression of the protooncogene ckit in normal and tumor tissues from colorectal carcinoma patients. If youve been diagnosed with a gastrointestinal stromal tumor gist, theres good news. It originates from the muscularis propria layer of the bowel. Colon excluding appendix, gastrointestinal stromal tumor. Gastrointestinal stromal tumor genetics home reference nih. Gastrointestinal stromal tumors gists are the most common mesenchymal tumors of the gastrointestinal gi tract.
Questions to ask about gastrointestinal stromal tumors. Gists arise in the smooth muscle pacemaker interstitial cell of cajal, or similar cells. However, in the colon excluding the rectum, it is exceedingly rare and comprises no more than 12% of all gists. In this sense, another case of colonic gist arising in a duplication cyst has been recently described 5. Berzin2, 1department of internal medicine, fairview hospital, cleveland clinic, cleveland, oh, usa, 2center for advanced endoscopy, division of gastroenterology, beth israel deaconess medical center and. They rarely occur in the colon, rectum and esophagus.
Gastrointestinal manifestations of endometriosis are fairly common, occurring in 512% of endometriosis patients 3. The most common locations of the disease in the gastrointestinal tract are the rectum 50%, sigmoid colon 1847%, ileumsmall intestine 25% and appendix 318% but it can be present in other locations as well 36. He rshey medical center gastrointestinal stromal tumors gist mesenchymal tumors of the. Gastrointestinal stromal tumors gist are usually found on the stomach or small intestine, but they can be found anywhere in or near the gi tract. Because gists usually involve the outer muscular layer, they have a propensity for exophytic growth. Gists are usually slow growing lesions, and are often incidentally discovered.
Your story matters citation wadhwa, vaibhav, eoin slattery, sagar garud, saurabh sethi, helen wang, vitaliy y. These cells are part of the autonomic nervous system. Neoplasms of the colonic submucosa are rare in children. Imatinib therapy for a patient with metastasis of colonic. We present a case of gist complicat ing a c olonic interposition. Gastrointestinal stromal tumor gist is a rare tumor comprising 0. The surveillance epidemiology and end results seer database was queried from 1990 to 2009 using cs schema v0203. Synchronous ileal stromal tumor gist and colonic adenocarcinoma hsinhui chiu1, taichien huang2, yaowen liu3, tienlung ko4, and naikuan lu2 1 d iv s o nfg a tr elgy,p m mdc 2s u rg ey,3p ath olgy nd 4r ad io lk gn hspt abstract synchronous existence of gastrointestinal stromal tumor and colonic adenocarcinoma was rarely reported. Gastrointestinal stromal tumor early detection, diagnosis. Rna in situ hybridization rnaish of primary tumors revealed significant enrichment of these markers in a subpopulation of pdac cells at the tumor stroma interface, which suggested an important role of microenvironmental cafs in pdac cell heterogeneity. A gastrointestinal stromal tumor gist is a type of tumor that occurs in the gastrointestinal tract, most commonly in the stomach or small intestine.
Pdf colonic gastrointestinal stromal tumour presenting as. Gist may be noncancerous benign or cancerous malignant. Gastrointestinal stromal tumors gist is mesenchymal tumors arising from the interstitial cells of cajal pace maker cells of the gastrointestinal tract git. Here we report an interesting case of a 55yearold male, who presented with pain abdomen for 4 months duration. Gastrointestinal stromal tumors gist are the most common mesenchymal tumors of the gastrointestinal tract. The rarity of colonic gist was confirmed by our analysis, presenting an incidence of 0. Gastrointestinal stromal tumors gist are a type of soft tissue tumor that usually begin in specialized nerve cells in the wall of the stomach, intestines, or rectum, known as interstitial cells of cajal. Davila, md, facg associate professor of medicine penn state milton s. Mini gastrointestinal stromal tumors gists in the small. Abdominal belly pain a mass or swelling in the abdomen nausea and vomiting feeling full after eating only a small amount of food loss of appetite weight loss problems swallowing for tumors in the esophagus some tumors grow large enough to block the passage of food.
Case report extra luminal colonic gastrointestinal stromal. C180 cecum c182 ascending colon c183 hepatic flexure of colon c184 transverse colon c185 splenic flexure of colon c186 descending colon c187 sigmoid colon c188 overlapping lesion of colon c189 colon, nos note 1. May 11, 2009 gastrointestinal stromal tumors are the commonest mesenchymal tumors of the gastrointestinal tract, the stomach and small intestine are the favored sites of occurrence. Pdf gastrointestinal stromal tumors gists are the most common subset of gastrointestinal gi mesenchymal tumors of varying differentiation. The symptoms of gastrointestinal stromal tumor depend on the site and size of the tumor, and may include abdominal pain, gastrointestinal bleeding or signs of obstruction.
Multiple, malignant, rapidly progressing mini gist with rapid progression. Pathology outlines gastrointestinal stromal tumor gist. After discussion at tumor board, a decision was made to resect the primary colonic mass as well as the primary gist. The association of smallbowel gist and colorectal neoplasia has been described in only four case reports. We report 2 new cases of colonic gists localized at descending and sigmoid colon. Although colorectal gists can be small and found incidentally, the majority appear to be high risk and carry a significant likelihood of recurrent and metastatic disease. Case report endometriosis mimicking colonic stromal tumor vaibhav wadhwa1, eoin slattery2, sagar garud2, saurabh sethi2, helen wang3, vitaliy y. Surgery improved the survival rate, while chemotherapy did not. Gastrointestinal stromal tumors gists are mesenchymal neoplasms of the gastrointestinal gi tract and are thought to develop from the interstitial cells of cajal, innervated cells associated with the auerbach plexus. Gastrointestinal stromal tumors gists are softtissue sarcomas that can be located in any part of the digestive system. A case of diffuse infiltrating gastrointestinal stromal. Iccs are cells of the autonomic nervous system, the part of the nervous system that regulates body processes such as digesting food.
Departments of 1 gastroenterology, 2 digestive surgery, 3 pathology, and 4 foundation for research in digestive diseases fienad. Colonic gastrointestinal stromal tumors gists are very uncommon. Colonic gists are described as transmural tumors that involve the intraluminal and extraserosal surfaces of the colon. Gist of small bowel location and greater malignant potential. Histologically gists vary from spindle cell tumors to epithelioid and pleomorphic tumors. Notes colon excluding appendix and rectum including net c180, c182c189, c199, c209 c180 cecum c182 ascending colon c183 hepatic flexure of colon c184 transverse colon c185 splenic flexure of colon c186 descending colon c187 sigmoid colon c188 overlapping lesion of colon c189 colon, nos c199 rectosigmoid junction c209 rectum, nos note 1. The tumors are thought to grow from specialized cells found in the gastrointestinal tract called interstitial cells of cajal iccs or precursors to these cells. Colonic gist is a rare solid tumor, and the incidence is stable. The exact cause of a benign tumor is often unknown. Typically, the body is able to balance cell growth and division. Gastrointestinal stromal tumors gists are rare lesions that constitute the majority of mesenchymal tumors in the gastrointestinal tract. Gist is the most common mesenchymal tumor originated in the digestive tract. Tumor which differentiates along line of interstitial cells of cajal, the guts pacemaker cell.
Gastrointestinal stromal tumor gist know the signs and symptoms of gastrointestinal stromal tumors. Here are some questions you can ask your cancer care team to help you better understand your cancer diagnosis and treatment options. Introduction gastrointestinal stromal tumors gists, caused by interstitial cells of cajal, have been proven to be the most common. Gist s start in specialized nerve cells located in the walls of your digestive system. They are defined as tumors whose behavior is driven by mutations in the kit gene 85%, pdgfra gene 10%, or braf kinase rare. Pdf gastrointestinal stromal tumor gist associated with. Find out about risk factors, symptoms, tests to diagnose, prognosis, staging, and treatment for gastrointestinal stromal tumors. Gists start in specialized nerve cells located in the walls of your digestive system. Abstract gastrointestinal stromal tumors gists developing in the colon are rare, accounting for\5 % of all gists. Coexistence of gastrointestinal stromal tumour and colorectal. Gists are typically defined by the expression of ckit cd117 in the tumor cells, as these activating kit mutations are see.
Gastrointestinal stromal tumors gist are rare mesenchymal neoplasms of the gastrointestinal tract with a malignant potential and unpredictable behavior. Colonic gists were found to be more common in males with a slight male predominance. If you have been diagnosed with a gastrointestinal stromal tumor or are worried about it, you likely have a lot of questions. The nccn guidelines panel for cervical cancer screening endorses the following guidelines for the prevention and early detection of cervical cancer. In december 2009, the patient underwent a left hemicolectomy in addition to resection of the primary gist, which originated in the small bowel. Synchronous occurrence of ileal stromal tumor gist and colonic adenocarcinoma. Introduction colonic gastrointestinal stromal tumors gists are very uncommon. Find out how gists are tested for, diagnosed, and staged.
634 226 1426 361 874 522 1098 1052 182 1006 978 1063 836 880 1030 1133 422 525 731 1506 627 1245 1099 499 572 290 1066 479 766 318 467 54 934 1133 1073 1181 766 740 877 790 734 93 420 1201